Navigation Links
New model of muscular dystrophy provides insight into disease development
Date:8/27/2012

Muscular dystrophy is a complicated set of genetic diseases in which genetic mutations affect the various proteins that contribute to a complex that is required for a structural bridge between muscle cells and the extracellular matrix (ECM) that provides the physical and chemical environment required for their development and function. The affects of these genetic mutations in patients vary widely, even when the same gene is affected. In order to develop treatments for this disease, it is important to have an animal model that accurately reflects the course of the disease in humans. In this issue of the Journal of Clinical Investigation, researchers at the University of Iowa report the development of a mouse model of Fukuyama's muscular dystrophy that copies the pathology seen in the human form of the disease.

By removing the gene fukutin from mouse embryos at various points during development, researchers led by Kevin Campbell were able to determine that fukutin disrupts important modifications of dystrophin that prevent the muscle cells from attaching to the ECM. Disruption of the gene earlier in development led to a more severe form of the disease, suggesting that fukutin is important for muscle maturation. Disruptions in later stages of development caused a less severe form of the disease. In a companion piece, Elizabeth McNally of the University of Chicago discusses the implications of this disease model for the development of new therapies to treat muscular dystrophy.


'/>"/>
Contact: Jillian Hurst
press_releases@the-jci.org
Journal of Clinical Investigation
Source:Eurekalert

Page: 1

Related biology technology :

1. Isis And Collaborators Alleviate Disease In Animal Models Of Myotonic Dystrophy
2. NDIA, NTSA Will Be On Hand At Seventh Annual Capitol Hill Modeling and Simulation Expo, Set To Focus On Homeland Security, First Response
3. New life sciences R&D model based on real-time learning and data-sharing needed to sustain innovation
4. A new imaging system produces 3-D models of monuments using unmanned aircraft
5. MultiCell Technologies Announces Positive Preclinical Results for MCT-465 and MCT-485 in Primary Liver Cancer In Vitro Models
6. Newly-Published Study Compares SURFAXIN LS™ and CUROSURF® in Well-Established Model of Respiratory Distress Syndrome
7. Ahram Biosystems Announces Launch of the New Economy Model F1-12 of Its Palm PCR Series
8. SPX-106T Arrests Development of Atherosclerotic Plaques in Mouse Model of Cardiovascular Disease
9. Karyopharm Therapeutics Announces Multiple Presentations on Selective Inhibitors of Nuclear Export (SINE) in Solid and Hematologic Malignancy Models and Spontaneous Canine Cancers at the American Association of Cancer Research (AACR) Meeting
10. New Software from SRI Speeds Development of Genome-Scale Models for Drug and Fuel Research
11. A mouse model brings new perspectives on Lafora disease
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/19/2014)...   ISN has released its third annual ... publication series. With data aggregated from over eight ... within its online contractor management platform, ISNetworld, this three-part ... (KPI) statistics. The Contractor ... is available in two industry specific volumes: Oil ...
(Date:12/17/2014)... 2014 Achim Noack has been named ... Group. In this position, Noack will assist Jerry Stoller, ... develop marketing strategy and supervise global marketing management and ... has tremendous knowledge and experience in the crop protection ... experience and innovative thinking will be a great asset ...
(Date:12/17/2014)... Gene synthesis and bioengineering company ... into a technology access and services agreement with ... proprietary protein engineering technology, known as “ProteinGPS®” to ... extremely excited that the global leader ADM has ... bioengineering technology has now been validated through many ...
(Date:12/17/2014)... FRANCISCO, Calif. , Dec. 17, 2014 /PRNewswire/ ... of a Phase 2 study evaluating the investigational ... treatment of patients with severe, chronic itch who ... standard of care, including topical steroids and antihistamines. ... percent change in Visual Analog Scale (VAS) itch ...
Breaking Biology Technology:ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 2ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 3Achim Noack Joins Stoller Group as Global Vice President of Marketing and Administration 2ADM and DNA2.0 Enter Into Protein Engineering Technology Access and Service Agreement 2Velocity Pharmaceutical Development, LLC and Tigercat Pharma, Inc. Announce Phase 2 Results for VPD-737 in Patients With Chronic Pruritus 2Velocity Pharmaceutical Development, LLC and Tigercat Pharma, Inc. Announce Phase 2 Results for VPD-737 in Patients With Chronic Pruritus 3Velocity Pharmaceutical Development, LLC and Tigercat Pharma, Inc. Announce Phase 2 Results for VPD-737 in Patients With Chronic Pruritus 4
... Award-winning Palm Vein Recognition Device Helps BayCare ... Records and,Safeguard Patient Privacy, SUNNYVALE, Calif., ... Inc., a leading supplier of innovative computer ... security solutions,today announced that BayCare Health System, ...
... Sept. 23 Endocare, Inc.,(Nasdaq: ENDO ), ... of minimally invasive technologies for tissue and tumor,ablation, ... for the first time posted on its website ... prostate cancer, covering,primary, salvage and focal cryoablation, also ...
... NicOx,S.A. (Euronext Paris: COX) today announced it ... leading producer of two-piece capsules, for,the commercial ... is NicOx, lead investigational product and the ... class of,anti-inflammatory agents. The aim of this ...
Cached Biology Technology:Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 2Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 3Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 4Fujitsu PalmSecure and HT Systems' PatientSecure Selected by BayCare Health System to Protect Patient Confidentiality and Prevent Medical Identity Theft 5Best Practices Document on Cryoablation Posted on Website of American Urological Association 2NicOx Signs Agreement With Capsugel for the Commercial Manufacture of Naproxcinod Oral Capsules 2NicOx Signs Agreement With Capsugel for the Commercial Manufacture of Naproxcinod Oral Capsules 3NicOx Signs Agreement With Capsugel for the Commercial Manufacture of Naproxcinod Oral Capsules 4
(Date:12/10/2014)... RALEIGH, N.C. , Dec. 9, 2014  Valencell, ... announced it is seeing a staggering demand from its ... demand, the Company revealed, is not solely coming from ... industries as well. "A wearable is only ... a result, accuracy is the ultimate driver in long-term ...
(Date:12/10/2014)... , December 9, 2014 ... ITN,s BCARD Platform   ... enterprise meeting scheduling solutions for business-to-business (B2B) events, ... world leader in mobile near-field communication (NFC), Bluetooth ...      (Logo: http://photos.prnewswire.com/prnh/20140612/691055) , Jifflenow ...
(Date:12/3/2014)... Calif. , Dec. 2, 2014   ... globally deployed, innovative test solutions for military, aerospace, ... newest version of its successful TS-900 PXI ... performance and features of high-end systems to customers ... outstanding value compared to traditional ATE. ...
Breaking Biology News(10 mins):Wearable Technology Products Demand Highly Accurate Biometric Technology 2Jifflenow And ITN International Bring Cutting-Edge Badge Scanning Technology To B2B Events 2Jifflenow And ITN International Bring Cutting-Edge Badge Scanning Technology To B2B Events 3Marvin Test Solutions Brings New Capabilities to PXI-Based Semiconductor Test with TS-960 2Marvin Test Solutions Brings New Capabilities to PXI-Based Semiconductor Test with TS-960 3
... researchers from Michigan State University and the University of Cincinnati ... of Excellence for Parkinson,s disease grant. The grant, from ... National Institutes of Health, makes MSU,s College of Human Medicine ... Two new MSU researchers, Jack Lipton, Ph.D., and Caryl ...
... of invasive ants in northern Australia, finding black holes ... has recognised the outstanding work of some of its ... The CSIRO Medals are awarded each year to staff ... the organisation. Awarded since 1985, the CSIRO Medals have ...
... in human beings. This means laughing at others is also ... being laughed at causes some people enormous problems in their ... affects people in all cultures alike. What is the ... gelotophobia? One of the aims of a study published recently ...
Cached Biology News:MSU becoming center of excellence for Parkinson's research 2CSIRO medal winners 2CSIRO medal winners 3Fear of being laughed at crosses cultural boundaries 2Fear of being laughed at crosses cultural boundaries 3Fear of being laughed at crosses cultural boundaries 4
Ms anti-Cyclophilin D...
... to qualitatively detect EGFR ... paraffin-embedded (FFPE) tissue sections, ... cell preparations, using Chromogenic ... Fluorescent In Situ Hybridization ...
Mouse GM CSF Construction Kit provides antigen affinity purified polyclonal capture and tracer antibodies, and antigen standard for development of approximately ten microplate assays....
Many mammalian cell types and experimental situations create challenges for standard transfection experiments. The ViraPower Lentiviral Expression System overcomes these challenges with efficient...
Biology Products: